For Research Use Only. Not for use in diagnostic procedures.

Yeast

Greater than 90% as determined by SDS-PAGE.

20mM Tris-HCl based buffer, pH8.0

Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C. Repeated freezing and thawing is not recommended. Store working aliquots at 4 degree C for up to one week.

Small volumes of DHB4 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

81.69kD

hydroxysteroid 17-beta dehydrogenase 4

peroxisomal multifunctional enzyme type 2

Peroxisomal multifunctional enzyme type 2

EDH17B4; SDR8C1; MFE-2; 17-beta-HSD 4; DBP; MPF-2??[Similar Products]

The protein encoded by this gene is a bifunctional enzyme that is involved in the peroxisomal beta-oxidation pathway for fatty acids. It also acts as a catalyst for the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids. Defects in this gene that affect the peroxisomal fatty acid beta-oxidation activity are a cause of D-bifunctional protein deficiency (DBPD). An apparent pseudogene of this gene is present on chromosome 8. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, May 2014]

HSD17B4: Bifunctional enzyme acting on the peroxisomal beta- oxidation pathway for fatty acids. Catalyzes the formation of 3- ketoacyl-CoA intermediates from both straight-chain and 2-methyl- branched-chain fatty acids. Defects in HSD17B4 are a cause of D-bifunctional protein deficiency (DBPD). DBPD is a disorder of peroxisomal fatty acid beta-oxidation. Defects in HSD17B4 are the cause of Perrault syndrome (PRS). PRS is a sex-influenced disorder characterized by sensorineural deafness in both males and females and ovarian dysgenesis in females. Some patients also have neurologic manifestations, including mild mental retardation and cerebellar and peripheral nervous system involvement. Belongs to the short-chain dehydrogenases/reductases (SDR) family.

Protein type: Cell development/differentiation; EC 1.1.1.n12; EC 4.2.1.107; EC 4.2.1.119; Lipid Metabolism - primary bile acid biosynthesis; Lyase; Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 5q23.1

Cellular Component: membrane; peroxisomal matrix; peroxisomal membrane; peroxisome

Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity; 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase activity; long-chain-enoyl-CoA hydratase activity; protein homodimerization activity; receptor binding

Biological Process: androgen metabolic process; bile acid biosynthetic process; estrogen metabolic process; fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA oxidase; osteoblast differentiation

Disease: D-bifunctional Protein Deficiency; Perrault Syndrome 1

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